Harsha Panchal

Review of clinical profile and bacterial spectrum and sensitivity patterns of pathogens in febrile neutropenic patients in hematological malignancies: A retrospective analysis from a single center.

Background: The aim of this study was to study clinical profile with bacterial spectrum and susceptibility patterns of pathogens in culture positive febrile neutropenic (FN) patients of hematological malignancies. Materials and Methods: We retrospectively reviewed the medical records of 23 hematological malignancy patients admitted with chemotherapy induced febrile neutropenia with culture positive results, at our institute between June 2011 and December 2011. Results: A total of 23 patients were studied 12 males and 11 females, with a median age of 35 years. Most common diagnosis was acute leukemia (78%) with the majority of patients developing febrile neutropenia during the induction phase of treatment. Most common presenting symptoms were fever, cough, dyspnea, and diarrhea. Primary site of infection was not found in 47% of patients while the rest had lung, gastro-intestinal and skin/soft-tissue infection. Overall 23 organisms were isolated during the study period, from blood (56%), sputum (46%), stool (23%), and nasal swab from one patient. Gram negative bacteria accounted for 78% of organisms while gram positive organisms accounted for 22% of the total isolates. The most common organisms were: Escherichia coli (43%), Staphylococcus aureus (22%), Pseudomonas aeruginosa (17.4%) and Klebsiella pneumonia (17.4%). Antibiotic sensitivity patterns of these bacteria were studied. Gram negative bacterial infections were associated with higher mortality (89%). Conclusions: Induction phase of treatment in acute leukemia is the major cause of FN in hematological malignancies at our institute and gram negative organisms are the predominant organisms with E. coli as major isolate while S. aureus represents the most common gram positive organism. Amikacin and cefoperazone/sulbactum appears to be initial antibiotic appropriate to cover most gram negative pathogens while vancomycin to be added for suspected gram positive infections. FN represents a major cause of morbidity and mortality in hematological malignancy patients, high index of suspicion and early empirical antibiotics with supportive care are main interventions to reduce high mortality for these patients. Antibiotics should be modified according to culture sensitive report as soon as possible.

A rare complication in a case of multiple myeloma on therapy with thalidomide and dexamethasone—Reversible posterior lobe leukoencephalopathy

Central nervous system involvement is an unusual manifestation of multiple myeloma. We report a case with a rare manifestation of multiple myeloma in the form of reversible white matter changes of occipital lobes presenting with blindness. This 45-year-old postmenopausal female presented to us (in March 2005) with complaints of lethargy, breathlessness on exertion and generalised bone pains of 3 – 4 months duration. General clinical examination was normal except presence of pallor and pedal edema. Systemic examination was also essentially normal except for presence of a threefinger palpable soft liver enlargement and a just palpable firm spleen. Investigations revealed the following picture: hemoglobin at 5 gm%, total leucocyte count of 5300 with a differential count of 60% polymorphs and 27% lymphocytes and no atypical cells, platelet count of 90000/c.mm. The ESR was 150 mm/hr. Biochemistry was normal except for a serum albumin level of 2.7 gm/dl and a globulin level of 5.3 gm/dl suggesting a reversed albumin/globulin ratio. Serum electrophoresis revealed a prominent monoclonal band. Quantitative serum immunoelectrophoresis revealed significantly high serum IgG levels of 2213 mg/dl (normal level1⁄4 650 – 1500 mg/dl) and a low IgM level of 50 mg/dl (normal level1⁄4 60 – 280 mg/dl). Serum beta-2-microglobulin level was normal (1.85 micro gm/ml). Ultrasound of the abdomen was suggestive of hepatomegaly with fatty infiltration and splenomegaly without alteration of echotexture. Bone marrow aspiration showed hypercellular marrow with infiltration by 95% atypical plasma cells. Conventional cytogenetic study showed normal XX karyotype. FISH study for deletion 13q was negative. Skeletal survey showed multiple punched out lytic lesions in bones. On routine screening, the patient was detected to be hepatitis B surface antigen positive. Considering the diagnosis as multiple myeloma stage III A, she was put on combination therapy with thalidomide and high dose dexamethasone every 28 days (thalidomide at a dose of 200 mg/day and dexamethasone at a dose of 40 mg/day from day 1 to day 4, day 9 to day 12 and day 17 to day 20 on even cycles and day 1 to day 4 only on odd cycles), along with cotrimoxazole prophylaxis and lamivudine for hepatitis B. After two such cycles she had a very good response with improvement in symptoms, resolution of anemia and thrombocytopenia with normalization of hemoglobin and platelets. Serum protein levels and electrophoresis became normal. Repeat quantitative immunoelectrophoresis was also normal. The dose of thalidomide was increased to 300 mg/ day in the third cycle. When she came for fourth cycle she was having sensory symptoms in lower limbs without evidence of motor weakness, suggestive of

Successful Allogeneic Hematopoietic Stem Cell Transplantation of a Patient Suffering from Type II Congenital Dyserythropoietic Anemia A Rare Case Report from Western India

The most frequent form of congenital dyserythropoiesis (CDA) is congenital dyserythropoietic anemia II (CDA II). CDA II is a rare genetic anemia in humans, inherited in an autosomally recessive mode, characterized by hepatosplenomegaly normocytic anemia and hemolytic jaundice. Patients are usually transfusion-independent except in severe type. We are here reporting a case of severe transfusion-dependent type II congenital dyserythropoietic anemia in a 5-year-old patient who has undergone allogeneic hematopoietic stem cell transplantation (HSCT) at our bone marrow transplantation centre. Patient has had up until now more than 14 mL/kg/month of packed cell volume (PCV), which he required every 15 to 20 days to maintain his hemoglobin of 10 gm/dL and hematocrit of 30%. His pre-HSCT serum ferritin was 1500 ng/mL and he was on iron chelating therapy. Donor was HLA identical sibling (younger brother). The preparative regimen used was busulfan, cyclophosphamide, and antithymocyte globulin (Thymoglobulin). Cyclosporine and short-term methotrexate were used for graft versus host disease (GVHD) prophylaxis. Engraftment of donor cells was quick and the posttransplant course was uneventful. The patient is presently alive and doing well and he has been transfusion-independent for the past 33 months after HSCT.

Primary vaginal myeloid sarcoma: a rare case report and review of the literature.

Myeloid sarcoma (chloroma, granulocytic sarcoma, or extramedullary myeloid tumour) is an extramedullary mass forming neoplasm composed of myeloid precursor cells. It is usually associated with myeloproliferative disorders but very rarely may precede the onset of leukemia. Here, we are presenting a rare case of primary vaginal myeloid sarcoma in a geriatric female patient without initial presentation of acute myeloid leukemia (AML). A 68-year-old female patient with ECOG Performance Score of 1 presented with pervaginal bleeding for 20 days. On colposcopic examination, she was found to have mass in the anterior fornix of vagina. A punch biopsy specimen revealed chloromatous infiltration of the vagina. LCA (leukocyte common antigen), MPO (myeloperoxidase), and c-kit were strongly positive on IHC (immunohistochemistry). The patients routine blood investigations were normal including peripheral smear, lactose dehydrogenase, uric acid, 2D echocardiography, conventional cytogenetics, bone marrow aspiration, and biopsy. The patient was given 4 cycles of decitabine (Decitex, manufactured by Sun Pharmaceutical Industries Limited, India), 20 mg/m2 for 5 days at an interval of 28 days. There was a partial response to decitabine according to RECIST criteria. As decitabine therapy was well tolerated, we are continuing in the same way until disease progression without any complications. The patient is undergoing regular follow-up at our centre.

Efficacy of single-dose rasburicase in the management of tumor lysis syndrome: a case series from a regional cancer center in western India

Background: Tumor lysis syndrome (TLS) is an oncological emergency. Rasburicase (recombinant urate oxidase) has been proven to be an effective therapy for prevention of TLS and its serious consequences in patients with hematological malignancies such as acute leukemias with high white blood cells count, Burkitt lymphoma, and lymphoblastic lymphoma with high tumor burden. The US Food and Drug Administration recommended daily dosing regimen for 5 days is unaffordable by each and every patient in developing countries such as India. Recently, the conducted studies have clearly shown a similar efficacy for a single dose of rasburicase. Herein, we report a case series of 15 patients, including children and adults with hematologic malignancies, in whom TLS was managed by a single dose of rasburicase. Materials and Methods: We retrospectively analyzed the efficacy of single-dose rasburicase (SDR) (0.15 mg/kg intravenous infusion over 30 min) in patients with hematologic malignancies at risk for TLS. The drug was administered in five adult and 10 pediatric patients admitted to the Gujarat Cancer and Research Institute between January 2013 and December 2014. Results: The study included 15 patients, out of which 10 were pediatric (8 male:2 female) and five were adults (5 male:0 female). Patients with hematologic malignancies having Eastern Cooperative Oncology Group performance status 0–2 and at high risk or potential risk for TLS were selected. The median ages in pediatric and adult groups were 7.7 years and 32 years, respectively. The presence of hyperuricemia (plasma uric acid (UA) levels ≥7.5 mg/dl) or a diagnosis of very aggressive lymphoma or leukemia based on the World Health Organization classification of hematopoietic and lymphoid neoplasms in patients was classified as high-risk. Rasburicase was administered in a single dose of 0.15 mg/kg intravenously over 30 min. Patients were evaluated by clinical examination and blood biochemical tests at frequent intervals. Plasma samples for UA were collected at baseline before rasburicase, 6–24 h post-rasburicase, 48 h post-rasburicase, and daily during treatment. The blood samples for UA during the course of treatment were collected in prechilled tubes containing heparin and immediately immersed and transported on ice. The blood samples were analyzed within 4 h of collection. Serum electrolytes, blood urea nitrogen, creatinine, calcium, and phosphorous were monitored daily during this period. A single dose of rasburicase produced a rapid and sustained therapeutic effect of lowering the plasma UA levels in all the 15 patients. Renal parameters normalized within 72 h. UA levels remained below 4 mg/dl throughout the administration of chemotherapy until discharge, and none of the patients required a repeat dosing of rasburicase. Conclusion: SDR is a highly economical and clinically effective way of managing patients with TLS and could serve as an alternative to the 5-day treatment in a resource-limited country such as India.

Sunitinib in metastatic renal cell carcinoma: Experience from single center study, efficacy and safety

BACKGROUND The 5-year survival rate for metastatic renal cell carcinoma (RCC) is estimated to be <10%. RCC is highly resistant to chemotherapy. Targeted agents are now first choice of therapy for metastatic RCC such as sunitinib and sorafenib. METHODS This study is a retrospective analysis of 15 patients having metastatic RCC treated with sunitinib. Apart from three patients, all had clear cell histology. Thirteen patients received dosage of 50 mg/d (4 weeks on/2 weeks off cycles). In 14 patients sunitinib was used as 1st line. The primary end point was objective response rate. Secondary end points were progression free survival (PFS) and safety. RESULTS Until date of reporting, 3 out of 15 patients are currently on sunitinib. The most common Memorial Sloan = Kettering Cancer Centre poor prognostic factor was an interval of <1 year between diagnosis and starting of treatment (80%). The objective response rate was 13.66% (complete response [CR] + partial response [PR] = 0 + 2). Clinical benefit rate (CR + PR + stable disease) was 60% (n = 9). Median PFS in this study was 7.5 months, with a range of 2-22 month. Median overall survival (OS) of patients in this study was 12 months with a range of 3-24 month. An impact of the dose or/and number of cycles on response was seen in this study, with patients having average cycles >3 showing better response rates, PFS and OS. Major toxicities seen were fatigue ( n= 7), diarrhea (n = 3) and skin rash (n = 4) with majority patients experienced Grade 1-2 toxicities. While Grade 3-4 toxicities include fatigue (n = 1), mucositis (n = 1) and nausea (n = 1). CONCLUSIONS These results confirm efficacy and safety profile of sunitinib in metastatic RCC, particularly as a first line. Sunitinib produced a 60% disease control rate for metastatic RCC in Indian patients, with acceptable rates of toxicity at a dose of 50 mg daily. Response rates were well matched to other studies confirming the efficacy of sunitinib.

Primary Hepatic Burkitt Lymphoma: A Bizarre Site and Triumph Tale

Primary hepatic Burkitt lymphoma (PHBL) is an extremely rare form extra nodal lymphoma and till now only 11 case reports have been found in the literature. We are reporting an adult female with primary hepatic Burkitts lymphoma, who achieved complete remission after 5 months of combination chemotherapy containing vincristine, cyclophosphamide, doxorubicin, methotrexate, prednisolone and intrathecal chemotherapy. She is under regular follow up at our institute.

Acquired severe aplastic anemia treated with antithymocyte globulin and cyclosporine: An experience of regional cancer center, Western India

Severe aplastic anemia (SAA) is a serious bone marrow disease that needs a comprehensive and service-intense treatment with either bone marrow transplantation (BMT) or immunosuppressive therapy (IST); both are difficult to optimally offer in resources-limited countries. Here, we report the outcome of IST using horse Antithymocyte globulin (ATG) in 18 (7 children; 11 adults) patients with SAA referred to our center in west India. Only 18 patients out of 102 diagnosed as AA in 2 years could receive IST, largely due to costs restraints. Although CR was seen in 30% in adults and 33% in pediatric cases, but overall 50% cases were able to enjoy transfusion-independence, requiring no further treatment. Treatment related mortality occurred in 6.2%, relapse in 6.2% and 6.2% had clonal evolution. This makes IST a valuable option for managing SAA in absence of bone marrow transplantation.

Paraneoplastic Recurrent Hypoglycaemic Seizures: An Initial Presentation of Hepatoblastoma in an Adolescent Male—A Rare Entity

Hepatoblastoma (HB) is a rare malignant tumour of the liver and usually occurs in the first three years of life. Hepatoblastoma in adolescents and young adults is extremely rare; nevertheless the prognosis is much worse than in childhood, because these kinds of tumours are usually diagnosed late. Characteristic imaging and histopathological and AFP levels help in the diagnosis of hepatoblastoma. Paraneoplastic features of hepatoblastoma are not uncommon at presentation and include erythrocytosis, thrombocytosis, hypocalcaemia, isosexual precocious puberty, and rarely hypoglycaemia. Even though hypoglycaemia is commonly seen in hepatocellular carcinoma, its association with hepatoblastoma is very rare. We present a case of 15-year-old male patient presenting with complaints of recurrent hypoglycaemic seizures ultimately leading to diagnosis of hepatoblastoma. Managed successfully with neoadjuvant chemotherapy, surgery and adjuvant chemotherapy with adriamycin and cisplatin based regimens. An extensive review of literature in the PubMed and MEDLINE did not reveal much data on paraneoplastic recurrent hypoglycaemic seizures as an initial presentation of hepatoblastomas in adolescents and young adults.

Primary Synovial Sarcoma of Kidney: A Rare Differential Diagnosis of Renomegaly

Synovial sarcomas (SS) are classified as subgroup of soft tissue sarcomas affecting mainly extremities of young adults. Primary SS of kidney are very rare tumours with poor prognosis. Though they have characteristic histology and immunohistochemistry (IHC) due to rarity of incidence it is difficult to diagnose them. Sometimes chromosomal rearrangement studies are required to confirm the diagnosis. We are presenting a case of 41-year-old male who was referred to our cancer centre for evaluation of left renal mass. CT scan of abdomen revealed a large left renal mass encasing the aorta. Biopsy of renal mass revealed poorly differentiated sarcoma and IHC was positive for vimentin, CD99, and BCL2 and negative for AE1, epithelial membrane antigen, and leukocyte common antigen. The patient was clinically inoperable as renal mass was encasing the aorta. So he was subsequently offered palliative chemotherapy in form of ifosfamide and adriamycin. CT abdomen shows partial response after 3 cycles of chemotherapy according to RECIST criteria.